von Willebrand Disease Questions & Answers

1. Sabih A, Babiker HM. Von Willebrand Disease. 2023 Jan. [QxMD MEDLINE Link]. [Full Text].

2. Veyradier A, Boisseau P, Fressinaud E, Caron C, Ternisien C, Giraud M, et al. A Laboratory Phenotype/Genotype Correlation of 1167 French Patients From 670 Families With von Willebrand Disease: A New Epidemiologic Picture. Medicine (Baltimore). 2016 Mar. 95 (11):e3038. [QxMD MEDLINE Link]. [Full Text].

3. Udvardy ML, Szekeres-Csiki K, Hársfalvi J. Novel evaluation method for densitometric curves of von Willebrand factor multimers and a new parameter (M(MW)) to describe the degree of multimersation. Thromb Haemost. 2009 Aug. 102(2):412-7. [QxMD MEDLINE Link].

4. [Guideline] Laffan MA, Lester W, O'Donnell JS, Will A, Tait RC, Goodeve A, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Aug 12. [QxMD MEDLINE Link]. [Full Text].

5. Casonato A, Galletta E, Daidone V. The elusive and heterogeneous pattern of type 2M von Willebrand disease: a diagnostic challenge. Eur J Haematol. 2018 Aug 6. [QxMD MEDLINE Link].

6. Sutherland MS, Cumming AM, Bowman M, et al. A novel deletion mutation is recurrent in von Willebrand disease types 1 and 3. Blood. 2009 Jul 30. 114(5):1091-8. [QxMD MEDLINE Link].

7. Odom B, Khourdaji I, Golas V, Zekman R, Rosenberg B. Acquired von Willebrand Disease Secondary to Clear Cell Renal Cell Carcinoma. J Endourol Case Rep. 2018. 4 (1):114-116. [QxMD MEDLINE Link]. [Full Text].

8. Leebeek FWG. New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome. Hemasphere. 2021 Jun. 5 (6):e586. [QxMD MEDLINE Link]. [Full Text].

9. Waldow HC, Westhoff-Bleck M, Widera C, Templin C, von Depka M. Acquired von Willebrand syndrome in adult patients with congenital heart disease. Int J Cardiol. 2014 Aug 1. [QxMD MEDLINE Link].

10. Ruth A, Meador M, Hui R, Loftis L, Teruya J. Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation Patients: A Single Institution's Experience. Pediatr Crit Care Med. 2019 Oct. 20 (10):980-985. [QxMD MEDLINE Link].

11. Heubner L, Trautmann-Grill K, Tiebel O, Mirus M, Güldner A, Rand A, et al. Treatment of Acquired von Willebrand Disease due to Extracorporeal Membrane Oxygenation in a Pediatric COVID-19 Patient with Vonicog Alfa: A Case Report and Literature Review. TH Open. 2023 Jan. 7 (1):e76-e81. [QxMD MEDLINE Link]. [Full Text].

12. Iarossi M, Hermans C. Acquired von Willebrand syndrome following a SARS-CoV2 infection. Acta Haematol. 2023 Feb 20. [QxMD MEDLINE Link].

13. Stuijver DJ, Piantanida E, van Zaane B, Galli L, Romualdi E, Tanda ML, et al. Acquired von Willebrand syndrome in patients with overt hypothyroidism: a prospective cohort study. Haemophilia. 2014 May. 20(3):326-32. [QxMD MEDLINE Link].

14. Pelland-Marcotte MC, Humpl T, James PD, Rand ML, Bouskill V, Reyes JT, et al. Idiopathic pulmonary arterial hypertension - a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children. Br J Haematol. 2018 Aug 23. [QxMD MEDLINE Link].

15. Cao XY, Li MT, Zhang X, Zhao Y, Zeng XF, Zhang FC, et al. Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center. J Clin Rheumatol. 2019 Dec 6. [QxMD MEDLINE Link].

16. Byams VR, Kouides PA, Kulkarni R, et al. Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres. Haemophilia. 2011 Jul. 17 Suppl 1:6-13. [QxMD MEDLINE Link].

17. Sanders YV, Giezenaar MA, Laros-van Gorkom BA, Meijer K, van der Bom JG, Cnossen MH, et al. von Willebrand disease and aging: an evolving phenotype. J Thromb Haemost. 2014 Jul. 12(7):1066-75. [QxMD MEDLINE Link].

18. [Guideline] Nichols WL, Hultin MB, James AH, et al, and the NHLBI von Willebrand Disease Expert Panel. The Diagnosis, Evaluation, and Management of von Willebrand Disease. Bethesda, Md: National Heart, Lung, and Blood Institute. NIH publication no. 08-5832. December 2007. [Full Text].

19. Rodeghiero F, Castaman G, Tosetto A. How I treat von Willebrand disease. Blood. 2009 Aug 6. 114(6):1158-65. [QxMD MEDLINE Link]. [Full Text].

20. Sanders YV, Fijnvandraat K, Boender J, Mauser-Bunschoten EP, van der Bom JG, et al. Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric-specific bleeding. Am J Hematol. 2015 Sep 16. [QxMD MEDLINE Link].

21. Tosetto A. Bleeding Assessment Tools: Limits and Advantages for the Diagnosis and Prognosis of Inherited Bleeding Disorders. Semin Thromb Hemost. 2016 Jul. 42 (5):463-70. [QxMD MEDLINE Link].

22. Mannucci PM. New therapies for von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 6. 2019 (1):590-595. [QxMD MEDLINE Link].

23. Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P, et al. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost. 2010 Sep. 8 (9):2063-5. [QxMD MEDLINE Link]. [Full Text].

24. Deforest M, Grabell J, Albert S, Young J, Tuttle A, Hopman WM, et al. Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease. Haemophilia. 2015 Sep. 21 (5):e384-8. [QxMD MEDLINE Link]. [Full Text].

25. Roberts JC, Flood VH. Laboratory diagnosis of von Willebrand disease. Int J Lab Hematol. 2015 May. 37 Suppl 1:11-7. [QxMD MEDLINE Link].

26. Baronciani L, Peyvandi F. How we make an accurate diagnosis of von Willebrand disease. Thromb Res. 2020 Dec. 196:579-589. [QxMD MEDLINE Link].

27. Sharma R, Haberichter SL. New advances in the diagnosis of von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 6. 2019 (1):596-600. [QxMD MEDLINE Link].

28. Goodeve A. Diagnosing von Willebrand disease: genetic analysis. Hematology Am Soc Hematol Educ Program. 2016 Dec 2. 2016 (1):678-682. [QxMD MEDLINE Link].

29. Hayward CP, Moffat KA, Graf L. Technological advances in diagnostic testing for von Willebrand disease: new approaches and challenges. Int J Lab Hematol. 2014 Jun. 36(3):334-40. [QxMD MEDLINE Link].

30. Flood VH, Abshire TC, Christopherson PA, Friedman KD, Cox Gill J, Montgomery RR, et al. Von Willebrand disease in the United States: perspective from the Zimmerman program. Ann Blood. 2018 Jan. 3:[QxMD MEDLINE Link]. [Full Text].

31. Mannucci PM. New therapies for von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 6. 2019 (1):590-595. [QxMD MEDLINE Link].

32. Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, et al. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22. 126 (17):2038-46. [QxMD MEDLINE Link]. [Full Text].

33. O'Donnell JS, Lavin M. Perioperative management of patients with von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 6. 2019 (1):604-609. [QxMD MEDLINE Link].

34. Franchini M, Targher G, Montagnana M, Lippi G. Antithrombotic prophylaxis in patients with von Willebrand disease undergoing major surgery: when is it necessary?. J Thromb Thrombolysis. 2009 Aug. 28(2):215-9. [QxMD MEDLINE Link].

35. O'Brien SH, Stanek JR, Kaur D, McCracken K, Vesely SK. Laboratory Monitoring During Pregnancy and Post-Partum Hemorrhage in Women with Von Willebrand Disease. J Thromb Haemost. 2019 Dec 5. [QxMD MEDLINE Link].

36. Miljic P, Noureldin A, Lavin M, Kazi S, Sanchez-Luceros A, James PD, et al. Challenges in the management of women with type 2B von Willebrand disease during pregnancy and the postpartum period: evidence from literature and data from an international registry and physicians' survey-communication from the Scientific and Standardization Committees of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2023 Jan. 21 (1):154-163. [QxMD MEDLINE Link]. [Full Text].

37. Neff AT. Current controversies in the diagnosis and management of von Willebrand disease. Ther Adv Hematol. 2015 Aug. 6 (4):209-16. [QxMD MEDLINE Link].

38. Di Paola J, Lethagen S, Gill J, et al. Presurgical pharmacokinetic analysis of a von Willebrand factor/factor VIII (VWF/FVIII) concentrate in patients with von Willebrand's disease (VWD) has limited value in dosing for surgery. Haemophilia. 2011 Sep. 17(5):752-8. [QxMD MEDLINE Link].

39. Vonvendi. U.S. Food & Drug Administration. Available at https://www.fda.gov/vaccines-blood-biologics/approved-blood-products/vonvendi. January 31, 2022; Accessed: March 31, 2023.

40. Leebeek FWG, Peyvandi F, Escobar M, Tiede A, Castaman G, Wang M, et al. Recombinant von Willebrand factor prophylaxis in patients with severe von Willebrand disease: phase 3 study results. Blood. 2022 Jul 14. 140 (2):89-98. [QxMD MEDLINE Link]. [Full Text].

41. Thomas VM, Abou-Ismail MY, Lim MY. Off-label use of emicizumab in persons with acquired haemophilia A and von Willebrand disease: A scoping review of the literature. Haemophilia. 2022 Jan. 28 (1):4-17. [QxMD MEDLINE Link].

Eleanor S Pollak, MD Associate Director of Special Coagulation, Associate Professor, Department of Pathology and Laboratory Medicine, Section of Hematology and Coagulation, University of Pennsylvania

Eleanor S Pollak, MD is a member of the following medical societies: American Society of Hematology, College of American Pathologists, National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi; Dova; Apellis; Pharmacosmos<br/>Serve(d) as a speaker or a member of a speakers bureau for: Sobi; Sanofi; Rigel.

Marcel E Conrad, MD (Retired) Distinguished Professor of Medicine, University of South Alabama

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Koyamangalath Krishnan, MD, FRCP, FACP Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Steven Stein, MD, Assistant Professor, Department of Medicine, Division of Hematology/Oncology, University of Pennsylvania

Steven Stein, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Hematology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

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